Classification of PH

Condensed clinical classification of pulmonary hypertension (updated from Simonneau et al.)

1. Pulmonary arterial hypertension (PAH)

1.1 Idiopathic
1.2 Heritable
1.2.1 BMPR2 mutation
1.2.2 Other mutations
1.3 Drugs and toxins induced
1.4 Associated with:
1.4.1 Connective tissue disease
1.4.2 HIV infection
1.4.3 Portal hypertension
1.4.4 Congenital heart disease (Table 6)
1.4.5 Schistosomiasis

1’. Pulmonary veno-occlusive disease and/or pulmonarycapillary haemangiomatosis

1”. Persistent pulmonary hypertension of the newborn

2. Pulmonary hypertension due to left heart disease

2.1 Left ventricular systolic dysfunction
2.2 Left ventricular diastolic dysfunction
2.3 Valvular disease
2.4 Congenital / aquired left heart inflow/outflow tract obstruction and congenital cardiomyopathies
2.5 Other

3. Pulmonary hypertension due to lung diseases and/or hypoxia

3.1 Chronic obstructive pulmonary disease
3.2 Interstitial lung disease
3.3 Other pulmonary diseases with mixed restrictive and obstructive pattern
3.4 Sleep-disordered breathing
3.5 Alveolar hypoventilation disorders
3.6 Chronic exposure to high altitude
3.7 Developmental lung diseases (Web Table III)

4. Chronic thromboembolic pulmonary hypertension and other pulmonary artery obstructions

4.1 Chronic thromboembolic pulmonary hypertension
4.2 Other pulmonary artery obstructions

5. Pulmonary hypertension with unclear and/or multifactorial mechanisms

5.1 Haematological disorders
5.2 Systemic disorders
5.3 Metabolic disorders
5.4 Others


2015 ESC/ERS clinical guidelines on the diagnosis and treatment of pulmonary hypertension:

Last updated: November 11, 2016